Amy Kunchok1, Eoin P Flanagan1, Karl N Krecke2, John J Chen3, J Alfredo Caceres4, Justin Dominick5, Ian Ferguson6, Revere Kinkel7, John C Probasco4, Miguel Ruvalcaba8, Jonathan D Santoro9, Kurt Sieloff10, Jeremy Timothy11, Brian G Weinshenker12, Andrew McKeon1, Sean J Pittock1. 1. Department of Neurology, Mayo Clinic, Rochester, MN, USA/Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA/Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA. 2. Department of Radiology, Mayo Clinic, Rochester, MN, USA. 3. Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA/Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA/Department of Ophthalmology, Mayo Clinic, Rochester, MN, USA. 4. Department of Neurology, John Hopkins University School of Medicine, Baltimore, MD, USA. 5. Division of Neurology, Sharp Rees-Stealy Medical Group, San Diego, CA, USA. 6. Department of Rheumatology, Yale University School of Medicine, New Haven, CT, USA. 7. Department of Neurology, University of San Diego, San Diego, CA, USA. 8. Department of Neurology, UC Davis Medical Center (UCDMC), Sacramento, CA, USA. 9. Division of Neurology, Children's Hospital Los Angeles, Los Angeles, CA, USA/Department of Neurology, Keck School of Medicine at University of Southern California, Los Angeles, CA, USA. 10. Department of Neurology, University of Michigan, Ann Arbor, MI, USA. 11. Department of Neurology, Wellspan Pediatric Neurology, Manchester, PA, USA. 12. Department of Neurology, Mayo Clinic, Rochester, MN, USA/Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA.
Abstract
BACKGROUND: The presence of co-existent neuronal antibodies (neuronal-IgG) in patients with myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG1) is not yet well understood. OBJECTIVES: The aim of this study was to investigate the co-existence of a broad range of neuronal-IgG in MOG-IgG1+ patients. METHODS: MOG-IgG1+ patients were tested for 17 neuronal-IgGs in cerebrospinal fluid (CSF) and serum including NMDA-R-IgG, AMPA-R-IgG, GABAB-R-IgG, LGI1-IgG, CASPR2-IgG, GABAA-R-IgG, GAD65-IgG, mGLUR1-IgG, DPPX-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1,2,Tr, and ANNA1,2,3. Clinical and radiological features of MOG-IgG1+ with NMDA-R-IgG in CSF were compared to a control cohort of MOG-IgG1+ patients without NMDA-R-IgG. RESULTS: A total of 376 MOG-IgG1+ patients underwent testing for neuronal-IgGs. Serum testing for neuronal-IgGs (113 adults, 142 children) identified one child with NMDA-R-IgG (0.7%), one child with CASPR2-IgG (0.7%), one adult with LGI1-IgG (0.9%) and one adult with GABAA-R-IgG (0.9%). CSF testing for neuronal-IgGs (97 adults, 169 children) identified seven children (4%) and seven adults (7%) with NMDA-R-IgG, and one adult with GABAA-R-IgG (1%). The MOG-IgG1+/NMDA-R-IgG+ patients had a median age of 17 (range: 2-39) years. Features associated with MOG-IgG1+/NMDA-R-IgG+ included encephalopathy (p = 0.001), seizures (p = 0.045), and leptomeningeal enhancement (p = 0.045). CONCLUSION: NMDA-R-IgG was the most frequently detected neuronal-IgG to co-exist with MOG-IgG1. MOG-IgG1+/NMDA-R-IgG+ patients most often presented with encephalopathy and seizures. Testing for MOG-IgG1 and NMDA-R-IgG may be warranted in patients with encephalopathy and inflammatory demyelinating syndromes.
BACKGROUND: The presence of co-existent neuronal antibodies (neuronal-IgG) in patients with myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG1) is not yet well understood. OBJECTIVES: The aim of this study was to investigate the co-existence of a broad range of neuronal-IgG in MOG-IgG1+ patients. METHODS: MOG-IgG1+ patients were tested for 17 neuronal-IgGs in cerebrospinal fluid (CSF) and serum including NMDA-R-IgG, AMPA-R-IgG, GABAB-R-IgG, LGI1-IgG, CASPR2-IgG, GABAA-R-IgG, GAD65-IgG, mGLUR1-IgG, DPPX-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1,2,Tr, and ANNA1,2,3. Clinical and radiological features of MOG-IgG1+ with NMDA-R-IgG in CSF were compared to a control cohort of MOG-IgG1+ patients without NMDA-R-IgG. RESULTS: A total of 376 MOG-IgG1+ patients underwent testing for neuronal-IgGs. Serum testing for neuronal-IgGs (113 adults, 142 children) identified one child with NMDA-R-IgG (0.7%), one child with CASPR2-IgG (0.7%), one adult with LGI1-IgG (0.9%) and one adult with GABAA-R-IgG (0.9%). CSF testing for neuronal-IgGs (97 adults, 169 children) identified seven children (4%) and seven adults (7%) with NMDA-R-IgG, and one adult with GABAA-R-IgG (1%). The MOG-IgG1+/NMDA-R-IgG+ patients had a median age of 17 (range: 2-39) years. Features associated with MOG-IgG1+/NMDA-R-IgG+ included encephalopathy (p = 0.001), seizures (p = 0.045), and leptomeningeal enhancement (p = 0.045). CONCLUSION: NMDA-R-IgG was the most frequently detected neuronal-IgG to co-exist with MOG-IgG1. MOG-IgG1+/NMDA-R-IgG+ patients most often presented with encephalopathy and seizures. Testing for MOG-IgG1 and NMDA-R-IgG may be warranted in patients with encephalopathy and inflammatory demyelinating syndromes.
Authors: Eoin P Flanagan; Shannon R Hinson; Vanda A Lennon; Boyan Fang; Allen J Aksamit; P Pearse Morris; Eati Basal; Josephe A Honorat; Nora B Alfugham; Jenny J Linnoila; Brian G Weinshenker; Sean J Pittock; Andrew McKeon Journal: Ann Neurol Date: 2017-02 Impact factor: 10.422
Authors: Dean M Wingerchuk; Brenda Banwell; Jeffrey L Bennett; Philippe Cabre; William Carroll; Tanuja Chitnis; Jérôme de Seze; Kazuo Fujihara; Benjamin Greenberg; Anu Jacob; Sven Jarius; Marco Lana-Peixoto; Michael Levy; Jack H Simon; Silvia Tenembaum; Anthony L Traboulsee; Patrick Waters; Kay E Wellik; Brian G Weinshenker Journal: Neurology Date: 2015-06-19 Impact factor: 9.910
Authors: Elia Sechi; Laura Cacciaguerra; John J Chen; Sara Mariotto; Giulia Fadda; Alessandro Dinoto; A Sebastian Lopez-Chiriboga; Sean J Pittock; Eoin P Flanagan Journal: Front Neurol Date: 2022-06-17 Impact factor: 4.086